Lipedema

Lipedema is a condition that is almost exclusively found in women[3] and results in enlargement of both legs due to deposits of fat under the skin.[2] Women of any weight may develop lipedema[2][3] and the fat associated with lipedema is resistant to traditional weight-loss methods.[4] There is no cure and typically it gets worse over time, pain may be present, and patients bruise easily.[2] Over time mobility may be reduced, and due to reduced quality of life, patients often experience depression.[3] In severe cases the trunk and upper body may be involved.[2] Lipedema is commonly misdiagnosed and is now becoming known as lipoalgia due to there being no edema.[5]

Lipedema
Other namesLipoedema, lipödem, lipalgia, adiposalgia, adipoalgesia, adiposis dolorosa, lipomatosis dolorosa of the legs, lipohypertrophy dolorosa, painful column leg, painful lipedema syndrome
A very advanced case of lipedema of the right leg (the knee is pointing to the right and is concealed by the overhanging lipedema).
SpecialtyVascular medicine
SymptomsIncreased fat deposits under the skin in the legs, increased extracellular fluid, inflammation,[1] easy bruising, pain[2]
CausesUnknown[2]
Risk factorsFamily member with the condition[3]
Differential diagnosisLipohypertrophy, chronic venous insufficiency, lymphedema[2]
TreatmentPhysiotherapy, exercise[2] compression stockings, emollients, liposuction[3]
FrequencyUp to 11% of women[2]

The cause is unknown but is believed to involve genetics and hormonal factors.[2] It often runs in families; having a family member with the condition is a risk factor for developing it.[2][3] Other conditions that may present similarly include lipohypertrophy, chronic venous insufficiency, and lymphedema.[2] It is estimated to affect up to 11% of women.[2] Onset is typically during puberty, pregnancy, or menopause.[2]

The fat associated with lipedema is shown to be resistant to weight loss methods; however, unlike other fat, lipedema is not associated with increased risks of diabetes or cardiovascular disease.[4] Physiotherapy may help to preserve mobility for a little longer than would otherwise be the case. Exercise, only as much as the patient is able to do without causing damage to the joints, may help with overall fitness but will not prevent progression of the disease.[2] Compression stockings can help with pain and make walking easier.[3] Regularly moisturising with emollients protects the skin and prevents it from drying out.[3] Liposuction to remove the fat can help if the symptoms are particularly severe.[3] While surgery can remove fat tissue it can also damage lymphatic vessels.[2] Treatment does not typically result in complete resolution.[6]

Presentation

Associated conditions

Depression and anxiety are very common for a variety of reasons, particularly the fact that diagnosis usually takes a long time and patients have received much advice on diet and exercise in the meantime, neither of which are effective treatment for the lipedema although they may help associated conditions.[7] Joint pain, arthritis, dry skin, fungal infections, cellulitis and slow wound healing are also associated with lipedema.[7]

Diagnosis

Differential diagnosis

[8][9][10][11][12][5]

LipedemaLipo-lymphedemaLymphedemaObesityVenous insufficiency/venous stasis
Symptoms:Fat deposits / swelling in legs and arms not in hands or feet; hands and feet may be affected as the disease progresses.Fat deposits / swelling widespread in legs/arms/torsoFat deposits / swelling in one limb including hands and feetFat deposits

widespread

Swelling near ankles; brownish discoloration of lower legs (hemosiderin deposits). Minimal swelling possible.
Male/female:FFF/MF/MF/M
Onset:Around hormonal shifts (puberty, pregnancy, menopause)Around hormonal shiftsAfter surgery that affects lymphatic system, or at birthAny ageAround onset of obesity, diabetes, pregnancy, hypertension
Effects of diet:Restricting calories ineffectiveRestricting calories ineffectiveRestricting calories ineffectiveDiets and weight loss strategies often effectiveNo relation to caloric intake
Presence of edema:Non-pitting edemaMuch edema; some pitting; some fibrosisPitting edemaNo edemaOften edema, but can also occur without edema in earlier stages
Presence of Stemmer Sign:Stemmer's Sign negativeStemmer's Sign positiveStemmer's Sign positiveStemmer's Sign negativeStemmer's sign may or may not be present in lymphedema/lipolymphedema
Presence of pain:Pain in affected areas likelyPain in affected areasNo pain initiallyNo painPain is likely
Affected population:Best estimate is 11% adult women (study done in Germany)Unknown; best estimate is a few percent of adult womenLow≥30% of US adults>30% of US adults
Presence of cellulitis:No history of cellulitisLikely history of cellulitisPossible history of cellulitisOften itching +/- discoloration mistaken for cellulitis
Family history:Family history likelyFamily history of lipedema likelyFamily history not likely unless primary lymphedemaFamily history likelyVery likely family history

Lipedema stages

Lipedema is classified by stage: Stage 1: Normal skin surface with enlarged hypodermis (lipedema fat). Stage 2: Uneven skin with indentations in fat and larger hypodermal masses (lipomas). Stage 3: Bulky extrusions of skin and fat cause large deformations especially on the thighs and around the knees. These large extrusions of tissue drastically inhibit mobility.[13][14]

Similar conditions

Lipedema is often underdiagnosed due to the difficulty in differentiating it from lymphedema, obesity, or other edemas.[15]

Lipo-lymphedema

Lipo-lymphedema, a secondary lymphedema, is associated with both lipedema and obesity (which occur together in the majority of cases), most often lipedema stages 2 and 3.[13]

Dercum's disease

Lipedema / Dercum's disease differentiation – these conditions may co-exist. Dercum's disease is a syndrome of painful growths in subcutaneous fat. Unlike lipedema, which occurs primarily in the trunk and legs, the fatty growths can occur anywhere on the body.[16][17]

Treatment

A number of treatments may be useful including physiotherapy and light exercise which does not put undue stress on the lymphatic system.[18] The two most common conservative treatments are manual lymphatic drainage (MLD) where a therapist gently opens lymphatic channels and moves the lymphatic fluid using hands-on techniques, and compression garments that keep the fluid at bay and assist the sluggish lymphatic flow.

The use of surgical techniques is not universal but research has shown positive results in both short-term and long-term studies[19][20] regarding lymph-sparing liposuction and lipectomy.[21]

The studies of highest quality involve tumescent local anesthesia (TLA), often referred to as simply tumescent liposuction. This can be accomplished via both Suction-Assisted Liposuction (SAL) and Power-Assisted (vibrating) liposuction.[10][22] The treatment of lipedema with tumescent liposuction may require multiple procedures. While many health insurance carriers in the United States do not reimburse for liposuction for lipedema, in 2020 several carriers regard the procedure as reconstructive and medically necessary and do reimburse.[23] Water Assisted Liposuction (WAL) is technically not considered to be tumescent but achieves the same goal as the anesthetic solution is injected as part of the procedure rather than before-hand. Developed by Doctor Ziah Taufig from Germany, it is usually performed under general anesthesia and is also considered to be lymph-sparing and protective of other tissues such blood vessels.[24]

Prognosis

There is no cure. Complications include a malformed appearance, reduced functionality (mobility and gait), poor Quality of Life (QOL), depression, anxiety, and pain.[5]

Epidemiology

According to an epidemiologic study by Földi E and Földi M, lipedema affects 11% of the female population, although rates from 6-39% have also been reported.[25][26]

History

Lipedema was first identified in the United States, at the Mayo Clinic in 1940.[27][28] Most attribute the original identification of lipedema to EA Hines and LE Wold (1951).[27] In spite of that lipedema is barely known in the United States to physicians or to the patients who have the disease. Lipedema often is confused with obesity or lymphedema, and a significant number of patients currently diagnosed as obese are believed to have lipedema, either instead of or in addition to obesity.[5]

See also

References
  1. Herbst KL, Kahn LA, Iker E, Ehrlich C, Wright T, McHutchison L, Schwartz J, Sleigh M, Donahue PM, Lisson KH, Faris T, Miller J, Lontok E, Schwartz MS, Dean SM, Bartholomew JR, Armour P, Correa-Perez M, Pennings N, Wallace EL, Larson E. Standard of care for lipedema in the United States. Phlebology. 2021 May 28:2683555211015887. doi: 10.1177/02683555211015887. Epub ahead of print. PMID 34049453.
  2. "Lipedema". rarediseases.info.nih.gov. Archived from the original on 18 March 2021. Retrieved 30 December 2016.
  3. "Lipoedema". nhs.uk. 2 October 2020. Retrieved 1 April 2021.
  4. Torre YS, Wadeea R, Rosas V, Herbst KL (March 2018). "Lipedema: friend and foe". Hormone Molecular Biology and Clinical Investigation. 33 (1). doi:10.1515/hmbci-2017-0076. PMC 5935449. PMID 29522416.
  5. Herbst, Karen L (2012). "Rare adipose disorders (RADs) masquerading as obesity". Acta Pharmacologica Sinica. 33 (2): 155–72. doi:10.1038/aps.2011.153. PMC 4010336. PMID 22301856.
  6. Anne Warren Peled, Anne; Kappos, Elisabeth (August 2016). "Lipedema: diagnostic and management challenges". International Journal of Women's Health. 8: 389–395. doi:10.2147/IJWH.S106227. PMC 4986968. PMID 27570465.
  7. Herbst, K. Rare adipose disorders (RADs) masquerading as obesity. Acta Pharmacol Sin 33, 155–172 (2012). https://doi.org/10.1038/aps.2011.153
  8. Fat Disorders Research Society Lipedema Description Archived 2015-07-31 at the Wayback Machine
  9. Todd, Marie (2010). "Lipoedema: Presentation and management". British Journal of Community Nursing. 15 (4): S10–6. doi:10.12968/bjcn.2010.15.Sup3.47363. PMID 20559170. S2CID 22897427.
  10. Fife, Caroline E.; Maus, Erik A.; Carter, Marissa J. (2010). "Lipedema". Advances in Skin & Wound Care. 23 (2): 81–92. doi:10.1097/01.ASW.0000363503.92360.91. PMID 20087075. S2CID 14350132.
  11. Földi, Michael; Földi, Ethel, eds. (2006). "Lipedema". Földi's Textbook of Lymphology. Munich: Elsevier. pp. 417–27. ISBN 978-0-7234-3446-7.
  12. Trayes, K. P.; Studdiford, J. S.; Pickle, S; Tully, A. S. (2013). "Edema: Diagnosis and management". American Family Physician. 88 (2): 102–10. PMID 23939641.
  13. Leopoldo Cobos, MD, Karen Herbst, PhD, MD, Christopher Ussery, MS, CSCS, MON-116 Liposuction for Lipedema (Persistent Fat) in the US Improves Quality of Life, Journal of the Endocrine Society, Volume 3, Issue Supplement_1, April–May 2019, MON–116
  14. Schmeller W, Hueppe M, Meier-Vollrath I. Tumescent liposuction in lipoedema yields good long-term results. Br J Dermatol. 2012;166(1):161‐168. doi:10.1111/j.1365-2133.2011.10566.x
  15. Buso G, Depairon M, Tomson D, Raffoul W, Vettor R, Mazzolai L (2019). "Lipedema: A Call to Action!". Obesity (Silver Spring). 27 (10): 1567–1576. doi:10.1002/oby.22597. PMC 6790573. PMID 31544340.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  16. Beltran K, Herbst KL. Differentiating lipedema and Dercum's disease. Int J Obes (Lond). 2017;41(2):240‐245. doi:10.1038/ijo.2016.205
  17. http://fatdisorders.org/fat-disorders/diagram%5B%5D
  18. Fetzer A, Wise C. Living with lipoedema: reviewing different self-management techniques. Br J Community Nurs. 2015;Suppl Chronic:S14‐S19. doi:10.12968/bjcn.2015.20.Sup10.S14
  19. Dadras, Mehran; Mallinger, Peter Joachim; Corterier, Cord Christian; Theodosiadi, Sotiria; Ghods, Mojtaba (2017). "Liposuction in the Treatment of Lipedema: A Longitudinal Study". Archives of Plastic Surgery. 44 (4): 324–331. doi:10.5999/aps.2017.44.4.324. PMC 5533060. PMID 28728329.
  20. Baumgartner, A.; Hueppe, M.; Schmeller, W. (May 2016). "Long-term benefit of liposuction in patients with lipoedema: a follow-up study after an average of 4 and 8 years". British Journal of Dermatology. 174 (5): 1061–1067. doi:10.1111/bjd.14289. PMID 26574236. S2CID 54522402.
  21. Sandhofer M, Hanke CW, Habbema L, et al. Prevention of Progression of Lipedema With Liposuction Using Tumescent Local Anesthesia: Results of an International Consensus Conference. Dermatol Surg. 2020;46(2):220‐228. doi:10.1097/DSS.0000000000002019
  22. Langendoen, S.I.; Habbema, L.; Nijsten, T.E.C.; Neumann, H.A.M. (2009). "Lipoedema: From clinical presentation to therapy. A review of the literature". British Journal of Dermatology. 161 (5): 980–6. doi:10.1111/j.1365-2133.2009.09413.x. PMID 19785610. S2CID 30001846.
  23. "Cosmetic and Reconstructive Services of the Trunk and Groin". November 12, 2019.
  24. Forner-Cordero, I.; Szolnoky, G.; Forner-Cordero, A.; Kemény, L. (2012). "Lipedema: An overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review". Clinical Obesity. 2 (3–4): 86–95. doi:10.1111/j.1758-8111.2012.00045.x. PMID 25586162. S2CID 45550292.
  25. Foldi, E. and Foldi, M. (2006) Lipedema. In Foldi's Textbook of Lymphology (Foldi, M., and Foldi, E., eds) pp. 417-427, Elsevier GmbH, Munich, Germany
  26. Reich-Schupke S, Schmeller W, Brauer WJ, et al. S1 guidelines: Lipedema. J Dtsch Dermatol Ges. 2017;15(7):758-767. doi: 710.1111/ddg.13036
  27. Wold, LE; Hines, EA; Allen, EV (1 May 1951). "Lipedema of the legs: a syndrome characterized by fat legs and edema". Annals of Internal Medicine. 34 (5): 1243–50. doi:10.7326/0003-4819-34-5-1243. PMID 14830102.
  28. HINES, EA (2 January 1952). "Lipedema and physiologic edema". Proceedings of the Staff Meetings of the Mayo Clinic. 27 (1): 7–9. PMID 14900206.
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